血管内大細胞型B細胞リンパ腫(intravascular large B-cell lymphoma : IVL) の知見補遺-Asian variant of IVLを中心とする文献的考察  [in Japanese] Clinicopathologic features of intravascular large B-cell lymphoma in Japan : review of the special reference to the Asian variant  [in Japanese]

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Abstract

Intravascular large B-cell lymphoma (IVL)の中で,Asian variant of IVL (AIVL)は血球貪食症候群により特徴付けられる。臨床的には,汎血球減少症,肝脾腫を呈し,腫瘤形成を認めることは稀である。Classical IVLの特徴である皮膚,神経症状に乏しく,生前診断は困難である。1990年以降,論文化された本邦のIVL 67例につき,AIVLの臨床的診断基準に合致する45例[Asian (A)群:平均年齢66歳;男性比62%]と,同基準に合致しない22例[Classical (C)群:65歳;73%]に分け,両群の病態を比較検討した。AIVLの組織学的診断基準の一つ,血球貪食像について記載のあった症例はA群に限られていた。骨髄浸潤,発熱,高ビリルリン血症,LDHおよびCRP高値はA群に有意に多かった。神経学的異常はC群に高頻度であった。皮膚病変はC群に限られていた。IVLはAIVL(A群)とclassical IVL(C群)の2つの臨床的亜群に大別されることが明らかとなった。

The Asian variant of intravascular large B-cell lymphoma (IVL) is characterized by hemophagocytic syndrome, i.e. the clinical features include pancytopenia, hepatosplenomegaly, and rarely mass formation. It usually lacks any neurological abnormality or skin lesions, which are typical features of classical IVL. Sixty-seven cases of IVL reported in Japan since 1990 were classified into two groups, and their clinicopathologic features were compared. Forty-five cases of IVL that met the clinical and laboratory criteria for the Asian variant were classified into Group A (average: 66 yr; male: 62%), and 22 cases that did not meet the criteria were classified into Group C (average: 65 yr; male: 73%). Hemophagocytosis was described only in Group A. The positivity rates for bone marrow invasion, fever, hyperbilirubinemia and elevated levels of LDH and CRP were significantly (p=0.0037, p<0.0001, p=0.0428, p=0.0108 and p=0.0008, respectively) higher in Group A than in Group C. On the other hand, the positivity rates for neurological abnormality and skin lesions were significantly (p<0.0001 and p=0.0011, respectively) higher in Group C than in Group A. Our reassessment of the reported cases of IVL in Japan identified two major categories of IVL: the classical form and the Asian variant.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 43(1), 5-11, 2002-01-30

    The Japanese Society of Hematology

References:  17

Cited by:  12

Codes

  • NII Article ID (NAID)
    10008355703
  • NII NACSIS-CAT ID (NCID)
    AN00252940
  • Text Lang
    JPN
  • Article Type
    Journal Article
  • ISSN
    04851439
  • NDL Article ID
    6293655
  • NDL Source Classification
    ZS21(科学技術--医学--内科学)
  • NDL Call No.
    Z19-295
  • Data Source
    CJP  CJPref  NDL  J-STAGE 
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